Masumoto K, Izaki T, Arima T
Department of Pediatric Surgery, Kitakyushu Municipal Medical Center, Japan.
Acta Paediatr. 2001 Jun;90(6):701-3.
Cornelia de Lange syndrome is known to be occasionally associated with gastrointestinal malformation. However, the occurrence of cecal volvulus in such anomalies is very rare. We report a 15-y-old Japanese boy with Cornelia de Lange syndrome associated with a cecal volvulus secondary to non-fixation of the cecum and ascending colon. The patient was admitted to our hospital because of abdominal pain and bilious vomiting. At emergent laparotomy, cecal volvulus with non-fixation of the ascending colon and cecum was diagnosed. Release of the cecal volvulus and the fixation of the cecum and ascending colon to the right retroperitonium were performed. The postoperative course was uneventful, except for sepsis caused by aspiration pneumonia soon after the operation.
Clinicians treating patients with this syndrome should be aware that such patients may be at risk for developing severe gastrointestinal anomalies, including cecal volvulus.
已知科妮莉亚·德朗热综合征偶尔会伴有胃肠道畸形。然而，此类异常中发生盲肠扭转的情况非常罕见。我们报告一名15岁的日本男孩，患有科妮莉亚·德朗热综合征，并发盲肠扭转，原因是盲肠和升结肠未固定。该患者因腹痛和胆汁性呕吐入院。在急诊剖腹手术中，诊断为盲肠扭转伴升结肠和盲肠未固定。进行了盲肠扭转松解术，并将盲肠和升结肠固定于右腹膜后。术后过程顺利，除术后不久因吸入性肺炎导致败血症外。
治疗该综合征患者的临床医生应意识到，此类患者可能有发生包括盲肠扭转在内的严重胃肠道异常的风险。
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